Neumann M A
J Neuropathol Exp Neurol. 1977 Mar-Apr;36(2):321-37. doi: 10.1097/00005072-197703000-00007.
A 41-year-old woman was hospitalized in 1939 with neurologic signs from which there was complete recovery over a two-year period. In 1965 she was readmitted because of increasing forgetfulness and inability to care for herself; there were no neurologic deficits. In June 1970 torticollis developed and persisted until her death 15 months later. No cerebellar signs were elicited, but increased muscle tone was prominent. The brain showed remarkable flattening of the inferior surface of the cerebellum; the pons was small. The medullary substance of the cerebellum and each branchium pontis appeared completely demyelinated. Transverse fibres were apparently absent in the central basis pontis. Microscopically in addition to the pontocerebellar atrophy there was degeneration of the vestibular system, reticular formation of the medulla oblongata and medial longitudinal fasciculi. The inferior olives showed only moderate neuronal degeneration in the caudal areas. Basal ganglia and thalamus showed some involvement in the pathological process. It is postulated that the vestibular-reticular system atrophy was the anatomic substrate for the torticollis, and apparently overshadowed cerebellar signs, which are ordinarily associated with pontocerebellar atrophy.
一名41岁女性于1939年因神经系统症状住院，两年内完全康复。1965年，她因记忆力减退和生活不能自理再次入院；当时无神经功能缺损。1970年6月出现斜颈并持续至15个月后死亡。未引出小脑体征，但肌张力增高明显。脑部显示小脑下表面明显扁平；脑桥较小。小脑的髓质和每个脑桥臂似乎完全脱髓鞘。脑桥中央基底部明显无横向纤维。显微镜下，除脑桥小脑萎缩外，还有前庭系统、延髓网状结构和内侧纵束变性。下橄榄核仅在尾部区域有中度神经元变性。基底神经节和丘脑在病理过程中也有一定受累。据推测，前庭-网状系统萎缩是斜颈的解剖学基础，且明显掩盖了通常与脑桥小脑萎缩相关的小脑体征。
